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Background: The current classification of pulmonary hypertension (PH) distinguishes between pre-capillary (PAWP ≤ 15 mmHg) and post-capillary (PAWP > 15 mmHg) forms, with left heart disease, especially heart failure with preserved ejection fraction (HFpEF), being a common cause of PH. We investigated the suitability of an HFpEF diagnosis instead of PAWP in diagnosing PH associated with HFpEF. Methods: In a retrospective, single-center analysis, we reviewed diagnoses from our database, focusing on patients initially diagnosed with idiopathic pulmonary arterial hypertension (IPAH) or PH associated with HFpEF (PH-HFpEF) based on their PAWP. These patients were reclassified, distinguishing between HFpEF and non-HFpEF cases. Patients with PH-HFpEF were further stratified by PAWP (≤15 mmHg or >15 mmHg). Results: The study included 350 patients: 214 (61.1%) with PAWP ≤ 15 mmHg and 136 (38.9%) with PAWP > 15 mmHg. Replacing the PAWP criterion with the HFpEF criterion resulted in the reclassification of 121 of 350 (34.6%) patients (115 of 214 [53.7%] from IPAH to PH-HFpEF and 6 of 136 [4.4%] from PH-HFpEF to IPAH). The final disposition was IPAH (n = 105, 30%), PH-HFpEF with PAWP ≤ 15 mmHg (n = 115, 32.9%), and PH-HFpEF with PAWP > 15 mmHg (n = 130, 37.1%). Characteristics such as age distribution, functional impairment, co-morbidities, echocardiographic indices of HFpEF, pulmonary vascular resistance, response to PH medications, and unadjusted survival were comparable between the two HFpEF cohorts but differed substantially from those with IPAH. Conclusions: PH-HFpEF patients with PAWP ≤ 15 mmHg resemble those with PAWP > 15 mmHg but differ from IPAH cases. Incorporating non-invasive HFpEF criteria could refine PH diagnostic classification. [ABSTRACT FROM AUTHOR]

Among patients meeting diagnostic criteria for idiopathic pulmonary arterial hypertension (IPAH), there is an emerging lung phenotype characterised by a low diffusion capacity for carbon monoxide (DLCO) and a smoking history. The present study aimed at a detailed characterisation of these patients. We analysed data from two European pulmonary hypertension registries, COMPERA (launched in 2007) and ASPIRE (from 2001 onwards), to identify patients diagnosed with IPAH and a lung phenotype defined by a DLCO of less than 45% predicted and a smoking history. We compared patient characteristics, response to therapy, and survival of these patients to patients with classical IPAH (defined by the absence of cardiopulmonary comorbidities and a DLCO of 45% or more predicted) and patients with pulmonary hypertension due to lung disease (group 3 pulmonary hypertension). The analysis included 128 (COMPERA) and 185 (ASPIRE) patients with classical IPAH, 268 (COMPERA) and 139 (ASPIRE) patients with IPAH and a lung phenotype, and 910 (COMPERA) and 375 (ASPIRE) patients with pulmonary hypertension due to lung disease. Most patients with IPAH and a lung phenotype had normal or near normal spirometry, a severe reduction in DLCO, with the majority having no or a mild degree of parenchymal lung involvement on chest computed tomography. Patients with IPAH and a lung phenotype (median age, 72 years [IQR 65–78] in COMPERA and 71 years [65–76] in ASPIRE) and patients with group 3 pulmonary hypertension (median age 71 years [65–77] in COMPERA and 69 years [63–74] in ASPIRE) were older than those with classical IPAH (median age, 45 years [32–60] in COMPERA and 52 years [38–64] in ASPIRE; p<0·0001 for IPAH with a lung phenotype vs classical IPAH in both registries). While 99 (77%) patients in COMPERA and 133 (72%) patients in ASPIRE with classical IPAH were female, there was a lower proportion of female patients in the IPAH and a lung phenotype cohort (95 [35%] COMPERA; 75 [54%] ASPIRE), which was similar to group 3 pulmonary hypertension (336 [37%] COMPERA; 148 [39%] ASPIRE]). Response to pulmonary arterial hypertension therapies at first follow-up was available from COMPERA. Improvements in WHO functional class were observed in 54% of patients with classical IPAH, 26% of patients with IPAH with a lung phenotype, and 22% of patients with group 3 pulmonary hypertension (p<0·0001 for classical IPAH vs IPAH and a lung phenotype, and p=0·194 for IPAH and a lung phenotype vs group 3 pulmonary hypertension); median improvements in 6 min walking distance were 63 m, 25 m, and 23 m for these cohorts respectively (p=0·0015 for classical IPAH vs IPAH and a lung phenotype, and p=0·64 for IPAH and a lung phenotype vs group 3 pulmonary hypertension), and median reductions in N-terminal-pro-brain-natriuretic-peptide were 58%, 27%, and 16% respectively (p=0·0043 for classical IPAH vs IPAH and a lung phenotype, and p=0·14 for IPAH and a lung phenotype vs group 3 pulmonary hypertension). In both registries, survival of patients with IPAH and a lung phenotype (1 year, 89% in COMPERA and 79% in ASPIRE; 5 years, 31% in COMPERA and 21% in ASPIRE) and group 3 pulmonary hypertension (1 year, 78% in COMPERA and 64% in ASPIRE; 5 years, 26% in COMPERA and 18% in ASPIRE) was worse than survival of patients with classical IPAH (1 year, 95% in COMPERA and 98% in ASPIRE; 5 years, 84% in COMPERA and 80% in ASPIRE; p<0·0001 for IPAH with a lung phenotype vs classical IPAH in both registries). A cohort of patients meeting diagnostic criteria for IPAH with a distinct, presumably smoking-related form of pulmonary hypertension accompanied by a low DLCO, resemble patients with pulmonary hypertension due to lung disease rather than classical IPAH. These observations have pathogenetic, diagnostic, and therapeutic implications, which require further exploration. COMPERA is funded by unrestricted grants from Acceleron, Bayer, GlaxoSmithKline, Janssen, and OMT. The ASPIRE Registry is supported by Sheffield Teaching Hospitals NHS Foundation Trust, Sheffield, UK. [ABSTRACT FROM AUTHOR]

Background: The prevalence of mental disorders, particularly adjustment disorder (AD), major depressive disorder (MDD) and panic disorder (PD) is increased in patients with pulmonary arterial hypertension (PAH). However, it is unclear which pathogenic mechanisms determine their development and could therefore be targeted in prevention or therapeutic interventions. Here, we assessed metacognitions in a sample of PAH patients with and without MDD and PD. Moreover, we reconstructed the course of mental illnesses following the PAH diagnosis. Methods: Two hundred seventeen PAH patients were included in this cross-sectional study. The prevalence of AD was assessed retrospectively using DSM-V criteria. Current mental disorders were assessed using the structured clinical interview for DSM-V. Additionally, metacognitive beliefs and processes were assessed using established questionnaires (MCQ-30, AnTI). Results: Patients with an AD consecutive to the PAH diagnosis more frequently developed MDD (37.5 vs. 13.9%, p < 0.001) and PD (26.3 vs. 8.8%, p = 0.001) later on compared to PAH patients without a former AD. Moreover, patients with current MDD/PD displayed more dysfunctional metacognitions than those without current MDD/PD (p < 0.001). Patients with current MDD/PD in the context of former AD had more dysfunctional metacognitive worries and beliefs compared to patients with current MDD/PD without former AD (p = 0.009). Conclusion: Our results suggest that in the context of PAH, dysfunctional metacognitions are associated with MDD and PD. Therefore, a metacognitive approach to treat and prevent those mental illnesses seems promising and should be investigated in future studies. [ABSTRACT FROM AUTHOR]

Objective: Pulmonary hypertension (PH) is a chronic and progressive pulmonary vascular disease resulting in symptoms such as shortness of breath and fatigue and leading to death from right heart failure if not adequately treated. Chronic thromboembolic pulmonary hypertension (CTEPH) is a subgroup of PH characterized by obstruction or occlusion of pulmonary arteries by post-embolic fibrotic material. To date, few studies examined symptoms of depression and anxiety in patients with CTEPH, showing depression levels as high as 37.5%. However, none of the former studies used structured expert interviews. Methods: Mental disorders were diagnosed using the Structured Clinical Interview for DSM-5 (SCID). The prevalence of mental disorders in patients with CTEPH were compared to the prevalence in patients with pulmonary arterial hypertension (PAH) and the general German population. Quality of life (QoL) was measured with World Health Organization (WHO) Quality of Life questionnaire (short form). Factors associated with QoL were analyzed with linear regression and the diagnostic value of the Hospital Anxiety and Depression Scale (HADS) was evaluated using receiver operating characteristics (ROC) curve analysis. Results: Hundred and seven patients with CTEPH were included. Almost one-third of the patients (31.8%) had current psychological disorders. Panic disorder (8.4%), specific phobia (8.4%), and major depressive disorder (6.5%) were the most prevalent mental illnesses. The prevalence of panic disorders was higher in CTEPH compared to the German population while major depressive disorder was fewer in CTEPH compared to PAH. The presence of mental disorders had a major impact on QoL. Hospital Anxiety and Depression Scale discriminated depression and panic disorder reliably. Conclusion: Mental disorders are common in patients with CTEPH and associated with an impaired QoL. The HADS may be a useful screening tool for panic and depression disorders in patients with CTEPH. Further research on therapeutic strategies targeting mental disorders in patients with CTEPH is needed. [ABSTRACT FROM AUTHOR]

Introduction: Chronic thromboembolic pulmonary hypertension (CTEPH) is a potentially life-threatening condition associated with high morbidity and mortality. However, advances in medical, surgical and interventional treatment have markedly improved the outcome of patients with CTEPH. Additional factors potentially influencing quality of life (QoL) and outcome in CTEPH are yet to be defined. Child maltreatment is a major risk factor for unfavorable behavioral, mental as well as physical health outcomes and has been associated with decreased QoL. To date, no study assessed the impact of childhood trauma in patients with CTEPH. Methods: Patients with CTEPH were invited to complete the Childhood Trauma Questionnaire (CTQ). Data were compared to prevalence data from the German population. Mental well-being was assessed using the Hospital Anxiety and Depression Scale (HADS) and quality of life was measured using the WHO Quality of Life Questionnaire (WHOQOL). Furthermore, lifestyle factors and physical health parameters were studied. Logistic regression analysis was used to investigate a possible impact of child maltreatment on markers of disease severity. Results: One-hundred and seven patients with CTEPH completed the CTQ. These patients reported higher rates of emotional abuse and physical abuse and emotional neglect compared to the German population while rates of physical neglect and sexual abuse did not differ between patients and German population with prevalence of 20.6% for emotional abuse, 20% for physical abuse, 22% for emotional neglect, 46% for physical neglect, and 6% for sexual abuse in patients with CTEPH. Higher CTQ scores were associated with anxiety symptoms as well as negatively associated with QoL. No direct impact of childhood trauma on CTEPH severity was found. Conclusion: We found a higher rate of child maltreatment in patients with CTEPH in comparison to the German population. Correlations suggest moderate associations between CTQ scores and mental health and QoL. Child maltreatment had no significant impact on disease severity. Further investigation on proper interventions to support affected patients is needed. [ABSTRACT FROM AUTHOR]

Background/Objective: Child maltreatment is associated with increased risk of psychological consequences, contributes to morbidity and has long lasting effects on mental health and quality of life. Child maltreatment has not been assessed in patients with pulmonary arterial hypertension (PAH). We examined the prevalence of child maltreatment and determined their impact on disease severity in patients with PAH. Methods: A cross-sectional observational multicenter study at two PH centers in Germany was conducted. Patients with a confirmed diagnosis of PAH were given a self-administered questionnaire. Child maltreatment using the Childhood Trauma Questionnaire (CTQ), quality of life (QoL), anxiety, depression, and lifestyle factors were assessed and enhanced by clinical parameters 6-min walk distance (6MWD), WHO functional class (WHO FC), and serum levels of N-terminal fragment of pro-brain natriuretic peptide (NT-proBNP). Prevalence rates of child maltreatment were compared to the general population and impact of child maltreatment on disease severity was calculated by logistic regression analysis. Results: Two-hundred and seventeen patients, 71% female and a median age of 56 years were enrolled in this study. Patients with PAH had higher rates of emotional abuse and lower rates of physical neglect compared to the German population while rates of emotional neglect, physical abuse, and sexual abuse did not differ between patients and German population. Patients with any form of child maltreatment were more likely to be active smokers, had a worse QoL and more anxiety or depression. Moderate associations between child maltreatment, mental health, QoL, lifestyle factors and clinical parameters could be observed. Logistic regression analysis showed a significant impact of CTQ-total score on disease severity with an OR of 1.022 (95%-CI: 1.001–1.042, p = 0.035). Conclusion: We found a higher rate of child maltreatment in patients with PAH in comparison to the German population. Correlations suggest moderate associations between CTQ-scores and mental health as well as QoL. Child maltreatment had significant impact on disease severity. However, effects were moderate. We conclude that child maltreatment has effects on mental health and quality of life in patients with PAH and may have limited effect on disease severity. [ABSTRACT FROM AUTHOR]

Introduction: Data on burden of pulmonary arterial hypertension (PAH) are mostly limited to physical and clinical endpoints as well as quality of life. Research on employment, work productivity, and educational background is scarce. The aim of this study was to assess the impact of PAH on employment status and work productivity in Germany. Materials and Methods: In a multicenter cross-sectional survey, patients with PAH were surveyed in two large pulmonary hypertension referral centers in Germany. The survey contained questions on education, employment, work productivity and impairment (WPAI, also at the time of diagnosis), quality of life, and socioeconomic status. Additional data was assessed using clinical research database for 6-min walk distance (6MWD), WHO functional class, and N-terminal fragment of pro-brain natriuretic peptide. All patients provided written informed consent, and the institutional review board approved this study. Results: In total, 212 patients were surveyed (72% female; median, 57 years) approximately 6 years after initial PAH diagnosis. A total of 76% had an idiopathic PAH followed by hereditary and associated PAH (10% each). Employment at the time of diagnosis was 48% (34% full-time, 14% part-time), with productivity measured by a WPAI score of 6 points and decreased to 29% (21% full-time, 8% part-time) at the time of the survey (with a WPAI score of 2 points, p < 0.001). Logistic regression showed education and 6MWD as predictors for employment. Patients in moderate or high educational category had a 3.6- or 5.6-fold chance, respectively, of being employed (p = 0.025 and p = 0.019), and per 50-m increase of 6MWD, the odds of being employed were 1.2, p = 0.042. Conclusion: Patients with PAH had a reduced employment rate, which was influenced by education and 6MWD. There was a considerable loss of employment over the course of the disease. Employment should be one possible treatment goal in patients with PAH to provide social participation to this patient group. [ABSTRACT FROM AUTHOR]

Background/Objective: Covid-19 pandemic may affect mental health and quality of life (QoL) in patients with pulmonary arterial hypertension (PAH). We assessed changes in anxiety and depression, quality of life (QoL) and self-described impact of Covid-19 in patients with PAH during the Covid-19 pandemic. Methods: This study included 152 patients with PAH from two German referral centers. Anxiety and depression were assessed using the Hospital Anxiety and Depression Scale (HADS-A and HADS-D) at two different timepoints before and during the Covid-19 pandemic with a median of 232 days between baseline and follow-up. QoL was assessed using EQ-5D and emPHasis-10. Perceived impact of Covid-19 and related regulations and measures were assessed using a set of specific questions and statements. Results: More than two thirds of patients had an unsuspicious HADS-A and HADS-D. Median scores did not differ from baseline for both HADS-A and HADS-D (p = 0.202; p = 0.621). Overall, no significant changes in HADS-A or HADS-D categories from baseline to follow up were observed (p = 0.07; p = 0.13). QoL did not change between baseline and follow-up. The Covid-19 pandemic had little impact on access to medical care and established PAH therapy. Patients were in agreement with governmental measures and regulations and felt sufficiently safe. Conclusion: First waves of Covid-19 pandemic had little impact on anxiety, depression and QoL in patient with PAH. Established PAH therapy and access to medical care were not affected. Further studies on the impact of prolonged duration of the ongoing Covid-19 pandemic are needed. [ABSTRACT FROM AUTHOR]

Objective: Mental health may affect the quality of life (QoL) in patients with pulmonary arterial hypertension (PAH). However, mental disorders have not been systematically assessed in these patients. We examined the prevalence of mental disorders using structured interviews and determined their impact on QoL in patients with PAH. Methods: This study included 217 patients with PAH from two German referral centers. Psychiatric disorders were assessed using the structured clinical interview for DSM-V. QoL was assessed using the WHO Quality of Life questionnaire (short form). The diagnostic value of the Hospital Anxiety and Depression Scale was evaluated by receiver operating characteristic curve analysis. Results: More than one third of the patients had psychological disorders with current or past adjustment disorder (38.2%), current major depressive disorder (23.0%), and panic disorder (15.2%) being the most prevalent mental illnesses. About half of the patients with a history of adjustment disorder developed at least one other mental illness. The presence of mental disorders had a profound impact on QoL. The Hospital Anxiety and Depression Scale ruled out panic disorder and depression disorder with negative predictive values of almost 90%. Conclusion: Mental disorders, in particular adjustment disorder, major depression, and panic disorder, are common in patients with PAH and contribute to impaired QoL in these patients. The Hospital Anxiety and Depression Scale may be used as a screening tool for the most common mental health disorders. Future studies need to address interventional strategies targeting mental disorders in patients with PAH. [ABSTRACT FROM AUTHOR]

Methods: In this retrospective observational study, we analyzed all patients with pulmonary arterial hypertension undergoing LenusPro® pump implantation between November 2013 and October 2019 at our center. Periprocedural safety was assessed by describing all complications that occurred within 28 days after surgery; complications that occurred later were described to assess long-term safety. Clinical outcomes were measured by comparison of clinical parameters and echocardiographic measurements of right ventricular function from baseline to 6-months-follow-up.Results: Fifty-four patients underwent LenusPro® pump implantation for intravenous treprostinil treatment during the investigation period. Periprocedural complications occurred in 5 patients; the only anesthesia-related complication (right heart failure with recovery after prolonged intensive care and death in the further course) occurred in the only patient who underwent general anesthesia. All other patients underwent local anesthesia with or without short-acting (analgo-) sedation. Eighteen long-term complications occurred in 15 patients, most notably pump pocket or catheter related problems. Transplant-free survival rates at 1, 2, and 3 years were 77 %, 56 %, and 48 %, respectively.Conclusions: Subcutaneous pump implantation under local anesthesia and conscious analgosedation while avoiding intubation and mechanical ventilation is feasible in patients with advanced PAH. Controlled studies are needed to determine the safest anesthetic approach for this procedure.Background/objectives: Intravenous treprostinil treatment via a fully implantable pump is a treatment option for patients with advanced pulmonary arterial hypertension. However, there is no consensus on the preferred anesthetic approach for the implantation procedure. Primary objective was to assess periprocedural safety with particular attention to feasibility of local anesthesia and conscious analgosedation instead of general anesthesia. Long-term safety and clinical outcomes were secondary endpoints. [ABSTRACT FROM AUTHOR]